Spontaneous Bilateral Carotid Artery Dissection Revealing Multifocal Fibromuscular Dysplasia

Abstract

Background: Fibromuscular dysplasia (FMD) is a rare idiopathic segmental non-arteriosclerotic and non-inflammatory arterial disease of small to medium-sized vessels. Although it is considered a benign entity, FMD can lead to potentially severe cerebrovascular complications. We present an atypical clinical case of ischemic stroke (IS) caused by bilateral carotid dissection revealing multifocal FMD. Case Report: A 43-year-old right-handed female with no medical history suddenly developed somnolence with left-sided weakness. Clinical examination revealed left hemiplegia and left central facial paralysis with an NIHSS score: 22. Cerebral tomography (CT) with arterial angiogram revealed abilateralis of the middle cerebral arteries (MCA), a double dissection of the internal carotids (IC), aneurysmal ectasia of the left IC, and aneurysm of the M2 segment of the MCA. Brain magnetic resonance imaging (MRI) with arterial angiography confirmed the bilateral dissection. A cerebral angiography (CA) revealed a radiological pattern of multifocal FMD. The angiography of the aorta and its branches showed adiffuse dysplasia of the renal arteries. The patient was put under antiaggregants. The modified Rankin score (mRS) after three months was three. Conclusion: Cerebrovascular FMD has typical angiographic features, the "string of beads" appearance being the most common. Although the risk of a stroke in patients with cervical FMD is low, it can lead to a severe stroke and the long-term risks are unknown. Patient registries should be encouraged to better understand the mechanisms and evolutionary profile of FMD and alert clinicians to this potentially disabling condition.